Dysembryoplastic neuroepithelial tumors (DNTs) were first described in 1988. A DNT is a cortical tumor that produces enlargement of a gyrus, forming a megagyrus that exceeds the normal thickness of the cortex. This tumor is generally seen in young patients with a long-standing history of drug-resistant and disabling seizures mostly of the complex partial type. No neurological deficits are found between the interictal periods in the majority of the reported cases. A general agreement exists over the clinical and radiological manifestations of a DNT as well as the benign biological behavior. Two pathological variants of DNT have been recognized: the single form, composed only of the so-called specific glioneuronal element, and the complex form that additionally shows glial nodules and foci of cortical dysplasia. Nevertheless, and despite the benign biological course, nuclear atypias, cellular monstruosities, foci of necrosis and mitosis can also be found. Surgical eradication of the tumor will usuallly have a good prognosis, without recurrences and with a positive control to seizure-free clinical outcome. Controversy continues to exist over a hamartomatous or a neoplastic origin of this lesion. The DNT has been placed among the neuronal and mixed neuronal-glial neoplasms in the revised World Health Organization (WHO) brain tumor classification, a category that includes the gangliogliomas and the central neurocytomas. Ultrastructural studies and immunostaining techniques may suggest that these three lesions represent different spectrums of the same condition. This paper reviews the most recent publications to offer a better understanding of DNTs and their implications in diagnosis and management.